{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,8]],"date-time":"2026-09-08T11:28:54Z","timestamp":1788866934158,"version":"build-2803163510"},"reference-count":18,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2023,3,17]],"date-time":"2023-03-17T00:00:00Z","timestamp":1679011200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100000897","name":"Cystic Fibrosis Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000897","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2023,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>The effects of elexacaftor\u2010tezacaftor\u2010ivacaftor (ETI) on body composition in people with CF (pwCF) are unknown.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Dual\u2010energy X\u2010ray absorptiometry fat\u2010free mass and fat mass adjusted for height (FMI) as well as oral glucose tolerance test derived measures of insulin secretion and sensitivity were compared before and after ETI initiation in eight pwCF.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Patients median age: 22 years interquartile range (IQR: 16\u201328), 87.5% male, median time on ETI:11 months. Weight <jats:italic>z<\/jats:italic>\u2010score increased from \u22120.52 to 0.18 (<jats:italic>p<\/jats:italic>\u2009=\u20090.014); FMI increased from 4.12 to 6.29 (<jats:italic>p<\/jats:italic>\u2009=\u20090.014). Insulin secretion (C pep iAUC\/Gluc iAUC) increased from 8.71 to 14.21 (<jats:italic>p<\/jats:italic>\u2009=\u20090.021), insulin resistance (HOMA2 IR) increased from 0.73 to 1.25 (<jats:italic>p<\/jats:italic>\u2009=\u20090.014) and insulin sensitivity decreased (Matsuda) 8.88 to 5.58 (<jats:italic>p<\/jats:italic>\u2009=\u20090.036).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>ETI resulted in increased weight and fat mass. BMI and muscle mass did not change. Both insulin secretion and insulin resistance increased. Longer\u2010term metabolic consequences of ETI need further investigation.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ppul.26388","type":"journal-article","created":{"date-parts":[[2023,3,17]],"date-time":"2023-03-17T13:32:48Z","timestamp":1679059968000},"page":"1805-1811","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":30,"title":["The impact of elexacaftor\/tezacaftor\/ivacaftor on body composition in a small cohort of youth with cystic fibrosis"],"prefix":"10.1002","volume":"58","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5573-4656","authenticated-orcid":false,"given":"Andrea","family":"Granados","sequence":"first","affiliation":[{"name":"Department of Pediatrics Nicklaus Children's Hospital Miami Florida USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christine L.","family":"Chan","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Children's Hospital Colorado University of Colorado Anschutz Medical Campus Aurora Colorado USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9909-6724","authenticated-orcid":false,"given":"Amir","family":"Moheet","sequence":"additional","affiliation":[{"name":"Division of Diabetes, Department of Medicine, Endocrinology and Metabolism University of Minnesota Minneapolis Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Timothy","family":"Vigers","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Children's Hospital Colorado University of Colorado Anschutz Medical Campus Aurora Colorado USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ana Mar\u00eda","family":"Arbel\u00e1ez","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Washington University School of Medicine St Louis Missouri USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1086-9135","authenticated-orcid":false,"given":"Katie","family":"Larson Ode","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Iowa Stead Family Children's hospital Iowa City Iowa USA"},{"name":"Fraternal Order of Eagles Diabetes Research Center University of Iowa Iowa City Iowa USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2023,3,17]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1067\/mpd.2000.107891"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(19)32597-8"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2009.09.001"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clnu.2012.04.012"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.3390\/nu5062019"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00080312"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.109.1.45"},{"key":"e_1_2_9_9_1","unstructured":"BorchersHWPracma: Practical Numerical Math Functions 2022.https:\/\/CRAN.R-project.org\/package=pracma"},{"key":"e_1_2_9_10_1","unstructured":"Radcliffe Department of Medicine UoO. 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